Peripheral neuropathy in copper deficient Wilson′s disease due to excessive copper removal: a case report and literature review
10.3760/cma.j.issn.1006-7876.2019.11.009
- VernacularTitle: Wilson病过度驱铜致铜缺乏周围神经病一例并文献复习
- Author:
Jie ZHANG
1
;
Xuen YU
;
Wenlong AI
;
Yongguang SHI
;
Liangliang ZHANG
;
Bin SONG
;
Congbo SHA
;
Lin CHEN
;
Jiyuan HU
;
Renmin YANG
Author Information
1. Department of Neurology, Institute of Neurology, Anhui University of Traditional Chinese Medicine, Hefei 230061, China
- Publication Type:Journal Article
- Keywords:
Wilson disease;
Hepatolenticular degeneration;
Copper;
Peripheral neuropathy;
Neutropenia
- From:
Chinese Journal of Neurology
2019;52(11):919-924
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To report a case of peripheral neuropathy secondary to copper deficiency (CD) by long-term decoppering chelation in Wilson′s disease (WD) to enhance understanding of the disease, and to pay more attention to individualized treatment of WD.
Methods:A case of WD diagnosed 12 years ago confirmed by gene detection and since then treated with anti-copper agent was diagnosed as CD based peripheral neuropathy and significant neutropenia and followed up for six months, and the clinical manifestations, laboratory examination, electrophysiology, imaging features were summarized. The related literatures were reviewed.
Results:A total of 16 cases of WD complicated with CD were reviewed and analyzed, including seven males and nine females aged 13-56 years. All of them were treated with zinc for 1-38 years, and nine cases with peripheral neuropathy. Hematological indicators can be significantly improved and neurological symptoms can be partially alleviated after stopping copper removal treatment.
Conclusions:Peripheral neuropathy in a WD with treatment-related CD may occur in blind treatment, irregular treatment monitoring and without individualized treatment adjustment. It is necessary to monitor blood routine, copper and zinc metabolism regularly and advocate individualized treatment of WD.
